Welcome to Coleson's blog. We've created it to keep friends and family updated on the journey we are about to embark on.. We appreciate your support and words of hope and encouragement during this time when it matters most.

Thursday, May 26, 2016

New Surgery, New Plans, New Attitude

HOne of the hardest parts about this process is the complete loss of control over the well being of your child.  You get hit with this overwhelming information, just enough to fill you with anxiety but not enough to put all the plans in place or put you at ease.  Once you have all the information you start to take comfort in the "game plan".  I feel like that is where I am at today. 

We met with the neurosurgeon yesterday.  There was no denying once he threw up the pictures of the MRI that the cyst exists and is big.  Even to my untrained eye I knew immediately where it was and that it was big (I'm sure you will see it too in the picture below).  He explained the root cause of the cyst was due to a malformation of the skull which creates a "pooling" of spinal fluid in the base of his skull.  This in turn creates and imbalance of fluid flow along the spinal chord and brain.  Think of it as a river, and his skull in creating somewhat of a dam which affects the rest of the flow of the river (aka his spinal chord) which causes other "pools" to form along it.  Fortunately it is only the 1 and there are not others.  Bone malformation is one of the most common Hurler traits and is the reason for the hip surgery.  Hurler Syndrome is a "storage" disorder meaning substance (called gag) that should get processed and expelled by the body rather does not and instead stays in the body in places where there is room, mostly the joints.  Because this gag material is sitting in places where bones would normally develop, it causes the bones to develop abnormally.  It is a total body affect but each Hurler kid's bones develop different depending on where most of the gag goes in the body and in Coleson's case it had a big affect on the skull. 

It is big enough that they need to address it.  To fix it, they have to break down the dam so to speak.  So next Tuesday, the 31st, he will have surgery to remove some bone at the base of the skull  which will allow the fluid to stop pooling in the skull which naturally (over the coarse of a year) should reduce/eliminate the pooling in the spinal chord.  It is too dangerous to do surgery on the cyst itself as that would be require surgery on the spinal chord.  We will be back at the University of Minnesota for this surgery so back to our normal stomping grounds.  I asked if this surgery could be combined with a new port placement and was happy at least to hear that it could be and in fact is scheduled with the same general surgeon who put in in his central line 3 years ago.  It does mean another 6 hr. surgery and 4 night stay in the hospital but at least he will walk out of the hospital.  We have some follow up appts and MRIs over the next year but we should be able to plan the hip surgery for next summer. 

Though I was hoping he would not have any more "Major" surgeries after the hip surgery, I guess, technically, this isn't after the hip surgery.  It is unexpected and a risky procedure itself, but it allows Coleson another year to comprehend the hip surgery which may not be a bad thing.  It does mean another traumatic and scary experience for Coleson however and though I was hoping as he got older, he would just "get used" to all the pokes, prods, medicines, IVs, X-rays, EKGs, MRIs, echos, blood pressures, pulse ox..... you get my point, I think he is getting more afraid of them.  We will have to take extra care in tackling each procedure as simple and nonintrusive as it may seem to be. 

So we have some re-arranging of plans left to do but it also allows us to get home around mid-July instead of at the end of July so that we can get settled before school starts on August 1st. I do believe that everything happens for a reason, so while I still don't know the reason why kids are born with such difficulties, I do believe we were not yet ready for the hip surgery and needed another year to prepare.

After the appt. yesterday, we all went to Mall of America and spent 6 hrs. riding rides.  Paul and I even got to ride the adult rides for once since Grandma was with us watching the kids.  Peyton is still not big enough to ride those yet but maybe, just maybe by next year she will be.  She can't wait.  Coleson too loves the excitement of the stomach turning rides and unfortunately will have quite a few more years before he will be big enough to ride them. 

So think of last Tuesday as a trial run for this Tuesday and next year.  The outpouring of support has really kept me going these past couple of days and I cannot thank you all enough for that. 
The white is the pooling fluid and the "dam" where the skull meets the chord.

Tuesday, May 24, 2016

It's What's on the Inside that Counts!

I was hoping to start this entry with news of a successful surgery today but as one Hurler mother recently said, we need to be prepared to expect the unexpected.  I thought I had accounted for the unexpected by reserving a house for the summer but nothing prepared me for the events over the last 24 hrs. 

We had a relatively uneventful first couple days getting settled here in our temporary home.  Our place is nestled in a very nice neighborhood with a park 3 blocks away.  Our landlord is very accommodating and has a girl close in age to Peyton who only lives 1 block away.  Dairy Queen and a very nice local grocery store are within 3 blocks, the river across the street.  The weekend weather was gorgeous and without the distractions of every day life, we actually were able to relax on Sunday. 

The knots in my stomach didn't start to occur until we were on our way to the pre-op apt on Monday.  Coleson did well during the Child Life discussions and started out being a big boy for the x-rays but after 3 of them started to melt down and I had to help him through the other 4 or 5.  Our consult with Dr. Walker went really well and I was actually feeling very confident about the surgery.  The last item of the day was to get labwork.  We started off in the lab, which we found out could not do lab draws from his port.  We have not tried an non-sedated blood draw since he was first diagnosed and didn't even want to try.  So we went back up to the Dr.s office for a nurse to do the draw.  After about 15 minutes and several  pokes and prods, they were unable to get a blood return from the port.  There must either be clotted blood around it or a collapsed vein, regardless it needs to be replaced.  Next they brought in a PICU nurse to try an entry into a hand or foot.  By this time, Coleson had already had enough and even with 4 us holding him down, the nurse could not get a needle in a vein in either hand.  So they called in "Dee Dee" who for sure would be able to do it.  So again with the attempts but now on his feet.  Again we all had hands and legs secured and he was lying in my lap with me shielding his chest holding his head and he still wiggled and whaled rendering any attempt useless.  For a 4 year old with Hurler's he has superman strength!  We had to get the blood draw so they could get the right kind of blood (with the proper antibodies) prepared for today's surgery.  So Plan D was to use nitrous.  After 3 hrs and with the help of nitrous they finally got enough blood.  It was a horrible experience and not the way I wanted to start this surgical procedure. 

Today actually started off better, except for the complete lack of sleep for me, Coleson didn't mind the 5am wake up call and immediate bath with the special soap (which we also had to do last night as well).  He didn't start to get nervous until we were in the parking garage and refused to get out of the car.  However, once we got into the waiting room and play area, he was OK.  He was even OK up to the point that they masked him for his MRI.  Prior to the MRI, Dr. Walker said based on the X-rays that he consulted with a neurologist and they thought his neck was strong enough to perform the surgery as intended, with the femurs being done while he was facing down on his belly and then the hips with him lying on his back.  Last night we had talked about doing the entire procedure on his back, though a little more complicated for the surgeon, less risk for the patient.  This I felt was actually good news and was looking forward to getting this behind us.

We didn't receive the bad news until Dr. Walker came out after the MRI to give us an update.  I knew something was wrong when he wanted to talk to us privately.  He reassured us immediately that Coleson was OK but that the MRI revealed a cyst at the base of his brain on his spine.  This cyst was putting pressure on the spine and it would not be safe to perform the surgery.  The cyst, though currently was presenting no signs it was there externally, would need to be taken care of before it started to effect his balance and cognitive ability.  The next hour was overwhelming and all I wanted to do was to hold Coleson again but they wanted to perform more MRIs to ensure there were no other cysts.  Fortunately there was no more.  But now we are facing another surgery, plus we need to get the port replaced and who knows what will come of the leg/hip surgery. 

On the outside you would never know that Coleson even needed any surgery.  He climbs like a champ and even started doing flips in the pool.  He is super smart (ask him anything about the planets) and his communication is even maturing.  His latest IEP was good in every area.  But on the inside, you never know what exists and with a Hurler kid, it could be just about anything.  It's a total body condition and each kid presents differently.  So, another challenge for us and for him.

Two of my closest friends sent me this same message (separately coincidentally), "You never know how strong you are until being strong is the only choice you have."   I have never had a choice to be strong, it started when I was 4 and lost my mom.  I feel like life has challenged me in so many ways  it is the only choice I have and I will continue to be.  I've said this before but it remains true, I can only be this strong with the love and support of friends and family and yet again, you all pulled through for me today.  Our appt. with the neurosurgeon is tomorrow (today by the time you read this) and I'll hopefully have a better idea of what our new plans will be. 

Continue the prayers and wishes and this truly is hard on Coleson who knows enough to be scared but not enough to believe everything will be OK.  I really am concerned for his mental well being along with of course his physical well being.  I'll update as soon as we know more. 

Tuesday, May 3, 2016

Help Support MPS Research with Triple Match Now - 3 Weeks til Surgery

Its 3 weeks and counting until Coleson’s surgery.  Each day brings more and more anxiety.  I recently learned that a young girl who had the same surgery as Coleson cannot feel anything from her chest down.   Her surgery was more than 10 days ago.  This is the risk/reality that we face when we send our kids back to the operating room and wait for 6-8 hrs. and then even longer for them to wake up from sedation and try to wiggle their toes.   My heart sunk when I read the update and I cannot express the sorrow I feel for Brynlee, her brother and her parents.  I know they have not given up hope yet that she will regain feeling and so if you feel so inclined to pray or send a good thought to them, I know they could use the support.  There are quite a few families having the same surgery this summer, but I think Coleson is the next in line, and I know all of us are diligently waiting for updates as each one occurs.   Brynlee’s mother posted a saying, “Sometimes the best thing you can do is not think, not wonder, not imagine, not obsess.  Just breathe, and have faith that everything will work out for the best.”   If she can live by this, then I should be able to as well.


With that said, there is a great opportunity to support MPS research (something that doesn’t get a lot of attention) and have it TRIPLE MATCHED.   If you ever have thought about donating to MPS Research this the time, triple matches don’t come often!  Triple Match ends this Saturday.

Donating to #TeamMPS is easy! There are three ways to help us reach our $50,000 goal. Remember, all donations are triple matched, so a $10 donation becomes $30!

  1. Text the word "courage" with a donation amount to 41444.
  2. Donate through Penn Medicine to #TeamMPS. -  This is the best way so you can learn more about MPS and this
  3. Donate through PayPal.
    May 15th is MPS Awareness Day.  So please wear your purple in Coleson’s honor.   I’ll be updating this blog frequently over the next several months as we climb this next mountain.
Here is a flashback from 3 years ago of Peyton Singing the "Brave" song for Coleson during BMT.